Hereditary angioedema: An update

Abstract

Hereditary angioedema (HAE) is a rare, autosomal dominant disorder of C1 inhibitor (C1-INH) deficiency manifested with any combination of cutaneous angioedema (painless, nonpruritic, nonpitting swelling of the submucosal, dermal, or subcutaneous tissue), severe abdominal pain, or acute airway obstruction. HAE occurs in 1 in 50,000 individuals. [1] It is a potentially life-threatening disorder, with some studies reporting lifetime disease-associated mortality rates up to 33%, in hospitalized patients. [1] Any race can be affected, with no reported bias in different ethnic groups. [1